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  • 1
    ISSN: 1432-1076
    Keywords: Glycogenosis ; Granulocytopenia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 139 (1982), S. 295-296 
    ISSN: 1432-1076
    Keywords: Selenium ; Hair ; Infants ; Children
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Hair selenium content was estimated by instrumental neutron activation analysis in Libyan infants and children. There was no significant difference between the values of young infants from North and South Libya. The hair selenium content rapidly decreased from 1071 ±75 ng/g in newborns to 301±99 ng/g in 7–10 months old infants in North Libya and to 557±204 ng/g in South Libya. In North Libya the values of preschool children were 409±117 ng/g and those of school children 464±124 ng/g.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 139 (1982), S. 172-175 
    ISSN: 1432-1076
    Keywords: Maple syrup urine disease ; Acute phase of MSUD ; Insulin treatment
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract High and neurotoxic blood levels of leucine and its ketoanalogue develop in catabolic patients with maple syrup urine disease. The use of relatively high doses of insulin and additional glucose had a more pronounced effect on lowering leucine (and α-ketoisocaproate) blood levels than dietary elimination of leucine alone. This is demonstrated in 2 neonates after blood exchange transfusion and in one 4-months old patient suffering from febrile diarrhea.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Selenium ; Supplementation ; Plasma ; Glutathione peroxidase ; Glutathione S-transferase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The plasma glutathione peroxidase (GSHPx) activity was measured in normal adults and children and in patients with reduced selenium state because of dietary treatment of metabolic diseases (phenylketonuria or maple-syrup-urine disease) before and after selenium supplementation. Besides GSHPx (measured with t-butyl hydroperoxide, cumene hydroperoxide and hydrogen peroxide as acceptor substrates) the activity of glutathione S-transferase was estimated in plasma. Plasma GSHPx activity in healthy children was significantly lower than in healthy adults. In 11 dietetically treated patients with phenylketonuria or maple-syrup-urine disease the plasma GSHPx was reduced to about 17% of the values of healthy children of the same age. No glutathione S-transferase activity could be found in plasma of children in normal or reduced Se state. During administration of yeast rich in Se (200μg Se/d) for 90 days 2 healthy adults showed no significant change of plasma GSHPx activity. During Se supplementation (75–100μg Se/d) for 120–163 days 5 dietetically treated patients with PKU or MSUD exhibited a significant increase of plasma GSHPx activity within 2 days. The values reached a plateau after 1 to 3 weeks of supplementation and remained at this level within the following 4 to 5 months. Therefore, the activity of plasma glutathione peroxidase can be used as an indicator of short-term changes of selenium intake in selenium deficient individuals.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 138 (1982), S. 293-296 
    ISSN: 1432-1076
    Keywords: Maple syrup urine disease ; Blood exchange transfusion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two neonates with maple syrup urine disease were treated by exchange transfusion. Within 15 h blood leucine and KICA concentrations were lowered from 2.6 mM to 1.1 mM using 570 to 620 ml blood per kg body weight. The other branched-chain amino acid/keto acid pairs fell to normal. During exchange transfusion the patient's nitrogen balance seems to be negative. Further exchange transfusion was useless. More importantly the patient should be forced into an anabolic state by high caloric supply or insulin plus glucose treatment. More KICA than leucine was eliminated, however, KICA blood levels remained slightly higher than that of leucine indicating different leucine/KICA equilibria in extravascular compartments than in blood. In a given time interval exchange transfusion was more effective than peritoneal dialysis, probably due to a lack of an additional (peritoneal) membrane. Renal excretion of branched-chain amino and keto acids was very inefficient. The allegedly most toxic metabolite, KICA, had the lowest renal clearance of the branched-chain keto acids.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-1076
    Keywords: Selenium ; Glutathione peroxidase ; Selenium deficiency ; Selenium supplementation ; Nutrition
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The diets of 5 patients with phenylketonuria or maple-syrup-urine disease were supplemented with yeast which was rich in selenium. For 120 days the patients received 45 μg Se/day to increase the Se content of their diets to 10–12ng Se/Kjoule. Before supplementation the selenium content of serum (5–15 ng/ml) and whole blood (10–27 ng/ml), and the activity of the erythrocyte glutathione peroxidase (0.19–2.69 U37/g Hb), amounted to only 10–20% of normal. The serum selenium content reached normal values within 4 weeks of supplementation, followed by normalisation of the selenium content of whole blood within 4–8 weeks. Restoration of the activity of erythrocyte glutathione peroxidase took 9 to 15 weeks —the red cell life span. There was a significant positive correlation between the selenium content of the erythrocytes and the activity of erythrocyte glutathione peroxidase.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1076
    Keywords: Selenium ; Intake ; Children ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In 20 healthy infants and children, 5–20 months old, the Se intake was estimated by analysing food samples by instrumental neutron activation analysis. The intake was calculated by weighing the portions offered and actually consumed. The median Se content of the food amounted to 27 ng/g wet weight (gww) and median daily Se intake to 33.5 μg. The Se intake was not equally distributed over the day. About 50% of the daily Se intake was derived from the supper. The main Se sources (41%) for young children were cereal paps. Commercially, available meals (30 ng/g) contained less Se than home-made ones (50 ng/g). In nine dietetically treated patients with phenylketonuria the median Se intake amounted only to 6.9 μg/day corresponding to a mean Se content of the diet of 7.9 ng/g. The main Se source in the diet was vegetables (36.3%) and 20% derived from their protein supplements. The Se intake of young children, healthy or dietetically treated, cannot be calculated accurately from tables but must be estimated by measuring the Se content of the local food because cereals and vegetables-the main Se sources-exhibit great regional variations.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 142 (1984), S. 147-150 
    ISSN: 1432-1076
    Keywords: Homocystinuria ; 5,10-methylenetetrahydrofolate reductase deficiency ; Betaine ; Homocysteine remethylation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a 3-year-old mentally retarded girl with homocystinuria due to 5,10-methylenetetrahydrofolate reductase deficiency among different therapeutic approaches only treatment with betaine (15–20 g/day) resulted in a satisfactory biochemical response. Betaine improved homocysteine remethylation and thus lowered plasma homocystine to trace amounts and normalized the previously very low plasma methionine concentration. This biochemical response was associated with a clinical improvement although she remained mentally retarded.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1436-6215
    Keywords: nutrition ; children ; food ; fat ; lipid composition
    Source: Springer Online Journal Archives 1860-2000
    Topics: Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition , Medicine
    Description / Table of Contents: Zusammenfassung Die analytischen Methoden und die Ergebnisse der Analysen der Lipidbestandteile von käuflich erhältlicher Beikost für ältere Säuglinge und Kleinkinder werden beschrieben. Die Lipide wurden mit Äthanol/Zyklohexan extrahiert, chromatographisch in polare und nichtpolare Fraktionen getrennt; die Fettklassen (Triglyzeride, Monoglyzeride, freie Fettsäuren, Cholesterinester und Phospholipide) wurden getrennt und durch Vergleich mit Referenzsubstanzen und durch Gaschromatographie identifiziert bzw. bestimmt. Zur Bestimmung der Fettsäuren in der 2-Position der Triglyzeride wurden die Triglyzeride mit Pankreaslipase zu entsprechenden Monoglyzeriden hydrolysiert und die entstandenen Monoglyzeride gaschromatographisch getrennt. Die Linolsäure, 18.2ω6, wurde als vorherrschende Fettsäure in der 2-Position der Triglyzeride gefunden. Die anderen Fettsäuren wurden mit Ausnahme weniger gleichmäßig über die einzelnen Positionen verteilt. Das Fett machte 31 bzw. 48% der Gesamtkalorien der untersuchten Präparate aus. Vorausgesetzt, daß ein Gläschen pro Tag gefüttert wird, werden dadurch 0,6 bis 2,4% der Energie in Form von Linolsäure zugeführt.
    Notes: Summary Analytical methods and the results of the analysis of the lipids of commercially available canned supplementary foods for juniors are described. Lipids were extracted, separated into polar and non-polar fractions and the components (triglycerides, monoglycerides, free fatty acids, cholesterol ester, and phospholipids) were then identified and estimated, by comparison with reference substances and by gas chromatography. To determine the degree of unsaturation and positional distribution of the fatty acids, the triglycerides were hydrolyzed by pancreatic lipase to monoglycerides with the fatty acid in 2-position of glycerol, and the fatty acids and the monoglycerides were also separated by gas chromatography. Linoleic acid, 18.2ω6, was found as the predominant fatty acid in the 2-position of the triglycerides. The other fatty acids being distributed equally to all positions of glycerol with the exception of few minor fatty acids. Fat contributed to 31 to 48 percent of the total energy supply of the analyzed items; linoleic acid providing 0.4 to 2.4 percent of this energy assuming that one can of these products is consumed daily.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Graefe's archive for clinical and experimental ophthalmology 213 (1980), S. 207-213 
    ISSN: 1435-702X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Kaninchencornea und -conjunctiva werden genau 30 s lang mit 0,25 n NaOH verätzt. Anschließend wird der Bindehautsack mit 0,9% NaCl ausgespült. Die Hornhaut wird excidiert und Metabolite Glucose und Lactat im Hornhautstroma gemessen. Die Veränderungen der nutritiven Metabolite werden diskutiert.
    Notes: Abstract The cornea and conjunctiva of rabbits are burned with 0.25 n NaOH for exactly 30 s. Then, the cornea and conjunctiva are cleaned with 0.9% NaCl. After sacrification the cornea is rinsed and the levels of the glucose and lactate are measured. The alterations of the metabolites for corneal nutrition are discussed.
    Type of Medium: Electronic Resource
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